Congenital Heart Disease Huang et al

The potential of disproportionate growth of tricuspid valve after

decompression of the right ventricle in patients with pulmonary
atresia and intact ventricular septa
Shu-Chien Huang, MD,a Kozo Ishino, MD,b Shingo Kasahara, MD,b Ko Yoshizumi, MD,b
Yasuhiro Kotani, MD,b and Shunji Sano, MDb

Objective: Tricuspid valve size is the major determinant of outcomes for patients with pulmonary atresia with
intact ventricular septum. Lack of right ventricle–pulmonary artery continuity is associated with poor tricuspid
valve growth (decrement in Z-value). However, most reports did not show evidence for disproportionate growth
of the tricuspid valve after establishment of right ventricle–pulmonary artery continuity.
Methods: We studied 40 patients with pulmonary atresia with intact ventricular septum who underwent initial
right ventricular decompression for planned staged repair. The initial Z-value of the tricuspid valve diameter
(Zt1) was obtained from the echocardiography-derived normal value. The late Z-value (Zt2) was measured before
definitive repair or the last available Z-value, if definitive repair was not yet reached. The factors associated with
the changes of Z-values (Zt2 ! Zt1) were analyzed.
CHD

Results: The mean initial tricuspid Z-value (Zt1) was !6.2 " 3.5. After treatment (Zt2), the mean Z-value was
!6.0 " 3.4 (n ¼ 34). Overall, the tricuspid Z-values did not change. Individually, the change in Z-value (Zt2 !
Zt1) was larger than þ2 in 11 (32%) patients and smaller than !2 in 6 (18%) patients. Increases in Z-value
(Zt2 ! Zt1) were significantly associated with right ventricular pressure/left ventricular pressure ratio measured
after initial palliation (r ¼ !0.54; P ¼ .001) and the initial tricuspid valve Z-value (Zt1) (r ¼ !0.40; P ¼ .02).
Conclusions: Disproportional growth of the tricuspid valve can occur, especially in patients with small tricuspid
valves and lower right ventricular pressures after decompression. The findings support the possibility of neonates
with small tricuspid valves undergoing biventricular repair after right ventricular decompression surgery.

Pulmonary atresia with an intact ventricular septum
(PA–IVS) is an uncommon congenital heart disease with
a variable degree of right ventricular (RV) hypoplasia and
coronary artery anomalies. Patients with RV-dependent cor-
onary circulation, which contradicts decompression of the
RV, should be better managed with single ventricular circu-
lation (Fontan-type repair). Otherwise, achieving biventri-
cular repair with good functional status and low systemic
venous pressure is the ideal goal. For biventricular circula-
tion to be achieved, the tricuspid valve and RV must have
adequate size to support pulmonary blood flow.
From the Department of Cardiovascular Surgery,a National Taiwan University Hospi-
tal, Taipei, Taiwan, and the Department of Cardiovascular Surgery,b Okayama
University Hospital, Okayama University Graduate School of Medicine and
Dentistry, Okayama, Japan.
Shu-Chien Huang was supported by a grant from National Taiwan University Hospi-
tal, Taipei, Taiwan, for study in Okayama, Japan, from April 2006 to September
2006.
Received for publication Oct 24, 2008; revisions received Feb 26, 2009; accepted for
publication May 15, 2009.
Address for reprints: Shunji Sano, MD, Professor and Chairman, Department of Car-
diovascular Surgery, Okayama University Graduate School of Medicine, Dentistry
and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Okayama, 700-8558, Japan
(E-mail: s_sano@cc.okayama-u.ac.jp).
J Thorac Cardiovasc Surg 2009;138:1160-6
0022-5223/$36.00
Copyright ! 2009 by The American Association for Thoracic Surgery
doi:10.1016/j.jtcvs.2009.05.015
Previous studies of PA–IVS showed that RV and tricuspid
valve diameters had poorer growth when RV–pulmonary
artery continuity was not achieved.1-3 For better RV growth,
the initial RV outflow procedure (surgically or percutane-
ously) with or without systemic–pulmonary shunting is the
most commonly used repair strategy.4-7
There are many reports on increased RV volume after
surgical or catheter-based RV decompression procedures,
but there is a paucity of similar data describing the growth
of tricuspid valves.2,5,8-10
Inasmuch as a small tricuspid valve causes limited RV
inflow and the tricuspid valve diameter is considered the
determinant for patients to undergo single ventricle or bi-
ventricular repair, we reviewed the data at our institution
to see whether the tricuspid valve had ‘‘disproportionate
enlargement’’ compared with somatic growth after decom-
pression of the RV. We also wanted to identify the factors
associated with growth of the tricuspid valve after RV de-
compression surgery and reviewed the late follow-up echo-
cardiographic data to see whether the tricuspid valves
continued to grow after biventricular repair.
PATIENTS AND METHODS
Patient Population
Between March 1993 and June 2006, we identified 40 infants with a
diagnosis of PA–IVS who underwent RV decompression in our institution,

1160 The Journal of Thoracic and Cardiovascular Surgery c November 2009

Huang et al Congenital Heart Disease

and the incision in the pulmonary trunk. The ductus arteriosus was then li-
Abbreviations and Acronyms gated. The other 2 patients underwent percutaneous valvotomy and RV out-
flow tract patching as their initial treatments, respectively.
BSA ¼ body surface area
Secondary procedures. Diagnostic postoperative cardiac catheteri-
LVP ¼ left ventricular pressure zations were performed after the initial RV decompression surgery (n ¼ 33).
PA– IVS ¼ pulmonary atresia with intact The interval between operation and catheterization was 0.76 " 0.54 years.
ventricular septum At this stage, an RVP/LVP ratio greater than 0.5 was an indication for bal-
RV ¼ right ventricle (ventricular) loon pulmonary valvuloplasty (n ¼ 20). For patients with an RV end-dia-
stolic volume less than 50% of the predicted normal value estimated at
RVP ¼ right ventricular pressure
the last cardiac catheterization, we performed an ‘‘RV overhaul’’ procedure
t1 ¼ first time point (n ¼ 6).14,15 The RV overhaul procedure included repeat pulmonary valvot-
t2 ¼ second time point omy, transatrial and transpulmonary resection of the hypertrophied infun-
TV ¼ tricuspid valve dibular muscle, and adjustment of an interatrial communication. To
TVD ¼ tricuspid valve diameter increase blood flow through the tricuspid valve, we partially closed the atrial
septal defect, keeping right atrial pressure less than 15 mm Hg, and main-
Zt1 ¼ initial Z-value
tained a gradient across the atrial septal defect of less than 10 mm Hg.
Zt2 ¼ Z-value after treatment Definitive repair. We generally consider a tricuspid valve diameter
larger than 60% (Z ¼!6.3) of the normal mean as the indication for biven-
tricular repair. If the tricuspid valve is less than 50% of normal mean (Z
<!8.5), we consider single ventricle repair or one-and-a-half ventricle re-
and all were included in this study. Patients with critical pulmonary stenosis pair. The borderline cases (tricuspid valve size between 50% and 60% of

(pinhole patency of the pulmonary valve, suprasystemic pressures in the
RV, and duct-dependent pulmonary circulation) and intact ventricular septa
were also included (n ¼ 5) because, in all other respects, they were similar to
patients with PA–IVS. Patients with no detectable infundibulum or RV-
dependent coronary circulation did not undergo RV decompression and
thus were not included in this study.
Measurements
We reviewed the echocardiographic and cardiac catheterization data.
The tricuspid valve diameter was measured from echocardiograms at its
maximum in diastole from the apical 4-chamber view. This tricuspid valve
diameter was then expressed as a percentage of the normal mean and the
Z-score obtained with the formula from the echocardiography-derived
normal value calculated using body surface area11:
lnðTVDÞ ¼ 1:08 þ 0:4945$lnðBSAÞ (1)
where TVD ¼ tricuspid diameter (cm) and BSA ¼ body surface area (m2).
Z-value ¼ ðln½observed TVD( ! ln½mean normal TVD(Þ=0:08121 (2)
where TVD is as defined earlier.
Direct measurements obtained during catheterization were used to esti-
mate the RV/left ventricular systolic pressure (RVP/LVP) ratio. RV vol-
umes were calculated from biplane cineangiograms using the Simpson
rule, as previously described.12 RV end-diastolic volume was corrected
for BSA and was expressed as a percentage of the predicted normal using
the formula derived by Nakazawa and colleagues.13
Predicted normal RV end-diastolic volumeðmLÞ ¼ 75:1
! "1:43 (3)
3 BSA m2
Management of the Patients
All patients were treated with prostaglandin E1 infusion, and balloon
atrial septostomy was performed during cardiac catheterization in most of
the patients before surgery.
Initial procedures. Initial management was transarterial pulmonary
valvotomy and placement of a systemic–pulmonary shunt, without cardio-
pulmonary bypass, as previously reported14 for the majority (95%, 38/40)
of the patients. The pulmonary valve was penetrated and further opened
with a 3- or 4-mm Hegar dilator through the incision in the main pulmonary
artery; a polytetrafluoroethylene tube (Gore-Tex tube; W. L. Gore & Asso-
ciates, Inc, Flagstaff, Ariz) was anastomosed to the left subclavian artery
CHD
normal, Z ¼ !8.5 to !6.3) were judged on intraoperative findings. We
have the tendency to perform biventricular repair whenever possible. The
surgical procedure included construction of an unobstructed RV outflow
and closure of the intra-atrial communication and the systemic–pulmonary
shunt. We used various techniques including pulmonary valve repair, resec-
tion of obstructing infundibular muscle, excision of hypertrophied RV mus-
cle to enlarge the cavity, and monocuspid transannular patching to achieve
a normal-sized outflow tract. During biventricular repair or one-and-a-half
ventricle repair with a marginal tricuspid valve diameter, we used an adjust-
able stitch to close the final interatrial communication defect for better judg-
ment after weaning the patient from cardiopulmonary bypass. We left
a small fenestration during closure of the interatrial septum in 14 patients,
and 12 of them had spontaneous closure of the communication on follow-
up echocardiograms.
After definitive repair, the patients were monitored echocardiographi-
cally. Among the 27 patients who underwent biventricular repair, cardiac
catheterization data were available for 17 and follow-up echocardiographic
data were available for 24 patients.
Data Analysis and Statistical Methods
We compared the tricuspid valve Z-values at different time points to dis-
cern changes in tricuspid valve diameter. The first time point (t1) was de-
fined as the Z-value obtained from the initial echocardiogram. The second
time point (t2) was defined as the Z-value just before definitive repair if
the patient underwent definitive repair. Otherwise, we chose the Z-value
from the most recent echocardiogram among patients in whom definitive re-
pair was not completed (ie, those with mixed systemic and pulmonary cir-
culation). The third time point (t3) was defined as the Z-value from the most
recent echocardiogram among patients in whom biventricular repair was
completed. Disproportionate tricuspid valve growth was defined as the
change in tricuspid valve Z-values. Thus increase of Z-value indicated the
outgrowth of the tricuspid valve according to the BSA, and decrease of
Z-value indicated the ‘‘shrinkage’’ of the tricuspid valve to the BSA.
To examine whether lower RVP influenced the growth of the tricuspid
valve, we examined the relationship of RVP/LVP after initial palliation to
differences in Z-scores of tricuspid valves. After biventricular repair, the he-
modynamic data were reviewed and correlated with the size of the tricuspid
valve.
Results are presented as means " SDs or percentages. The data were an-
alyzed with SPSS software (SPSS Inc, Chicago, Ill). The paired Student t
tests were used for comparing continuous variables for the same patient.
The Wilcoxon signed rank test was used for comparison of continuous vari-
ables. Correlation was analyzed by linear correlation modeling, computing

The Journal of Thoracic and Cardiovascular Surgery c Volume 138, Number 5 1161
 Congenital Heart Disease Huang et al

the Pearson correlation coefficient (r) and nonparametric regression analysis TABLE 1. The factors analyzed for the change of tricuspid valve
(categorical variables), as appropriate. Z-value (Zt2–Zt1)
Pearson Univariate
RESULTS Variables correlation P value
Demographic Data Initial evaluation (t1)
Among the 40 patients (21 boys and 19 girls), the mean Body surface area !0.018 .92
age at the first operation was 28.6 " 17.2 days; the average Z-value of TV !0.40 .02
body weight was 3.0 " 0.6 kg. RV volume (% of normal) !0.32 .13
There were 2 deaths after the stage I operations, and 1 pa- Tricuspid regurgitation !0.05 .79
tient was lost to follow-up. Two patients were waiting for Presence of sinusoid !0.307 (Spearman rho) .099
their next operations after initial valvotomy and shunt place- Age at first operation !0.11 .53
Second evaluation (t2)
ment. The remaining 35 patients received further treatment
Age at evaluation (t2) 0.27 .13
as follows: Four patients received bidirectional Glenn
RV volume (% of normal) !0.10 .58
shunts, and 1 of these infants died. Two patients were wait- RVP/LVP !0.54 .0013
ing for definitive repair after RV overhaul, and 29 patients TV, Tricuspid valve; RV, right ventricular; RVP, right ventricular pressure; LVP, left
underwent definitive repairs. ventricular pressure.
Mean patient age at definitive surgery was 2.4 " 0.9
years. The definitive repairs included biventricular repair
CHD

(n ¼ 27), Fontan circulation (n ¼ 1), and one-and-a-half ven-
tricle repair (n ¼ 1). One late death after biventricular repair
occurred 6 months after surgery; the cause of death was not
clearly known (presumed arrhythmia). The overall median
follow-up period after the initial procedure was 6.8 years
(1–13 years).
Tricuspid Valve Growth
There were 34 patients with Z-values at both t1 and t2
available for analysis. The initial mean tricuspid valve Z-
value (Zt1) was !6.2 " 3.5, which corresponded to a Z-
value of !1.9 " 1.5 by autopsy-derived nomogram.16 After
treatment (Zt2), the Z-value was!6.0 " 3.4 (Figure 1). The
age at the t2 point was 2.2 " 1.1 years old. Overall, the
average of tricuspid valve Z-value did not change (P >
.05, paired Student t test). The RV volume increased
from 44% " 34% to 57% " 30% of the normal value
(P < 0.05).
Comparing the tricuspid valve Z-value after treatment
(Zt2) with initial Z-value (Zt1) for individual patients showed
that the change in Z-value (Zt2!Zt1) was larger thanþ2 in 11
(32%) patients and smaller than!2 in 6 (18%) patients. The
other 17 (50%) patients had Z-value changes within " 2. The
increases in Z-values (Zt2 ! Zt1) were not related to patient
age at initial operation, presence of sinusoid communication,
or severity of tricuspid regurgitation (Table 1) The increases
in Z-values (Zt2!Zt1) were significantly negatively associ-
ated with RVP/LVP measured after initial palliation
(Figure 2) (r ¼ !0.54; P ¼ .0013). The increases were also
more marked among patients with initially small tricuspid
valve Z-values (r ¼!0.40; P ¼ .02) (Figure 3).
Among the 27 patients in whom biventricular circulation
was achieved, the mean initial Z-value (Zt1) was!5.8 " 3.6,
and the mean Z-value was!4.9 " 2.63 (P<.05) when they
received biventricular repair. The distributions of tricuspid
valve size with regard to 50% and 60% of normal value

FIGURE 2. The change of Z-value (Zt2–Zt1) showed negative correlation

FIGURE 1. The tricuspid valve Z-value at the initial evaluation (t1) and with the right ventricular pressure/left ventricular pressure (RVP/LVP)
after treatment (t2). measured after initial palliation (P ¼ .0013).

1162 The Journal of Thoracic and Cardiovascular Surgery c November 2009

Huang et al
FIGURE 3. The change of Z-value (Zt2 – Zt1) showed negative correla-
tion with the initial tricuspid valve Z-value (P ¼ .02).
(proposed criteria for biventricular repair) in these 27 pa-
tients are depicted in Table 2. Among the 27 patients, 13
(48%) had initial Z-values less than !6.3, and 7 of them
had increased Z-values to more than!6.3 at the stage of de-
finitive repair.
Seventeen patients underwent follow-up cardiac catheter-
ization after definitive repairs (mean of 1.66 " 1.11 years af-
ter repair). Their right atrial pressures negatively correlated
with tricuspid valve Z-value (Pearson r ¼ !0.49; P ¼ .038)
(Figure 4). All of these patients had right atrial pressures of
less than 12 mm Hg, except for 1 patient with a right atrial
pressure of 14 mm Hg. This particular patient had a tricuspid
valve diameter equal to 48% of normal (Z-value ¼!9), but
she was considered a high-risk Fontan candidate because
of left pulmonary vein occlusion after reimplantation for
anomalous return. She was still alive at 4 years of follow-up.
Among the patients who underwent successful biventric-
ular repairs, tricuspid valve Z-values further increased an
average of 1.14 " 1.09 by 4.9 " 3.2 years after definitive
surgery (P < .001, n ¼ 24). We could not find any specific
parameter associated with the ‘‘enlargement’’ of the tricus-
pid valve after biventricular repair.
DISCUSSION
In this study, we found that the disproportionate growth of
the tricuspid valve existed in some patients with PA–IVS
and related to the small initial tricuspid valve diameter and
TABLE 2. The distribution of tricuspid valve size among the 27
patients who received biventricular repair
Tricuspid valve size
Z-value (% normal)
Timing of Z <!8.5 !8.5 ) Z <!6.3
echocardiography (<50%) (50%–60%)
Initial evaluation (t1) 6 7
At biventricular repair (t2) 2* 4 21
*The 2 patients have Z-values ¼ !10.2 and !9.7, respectively.
The Journal of Thoracic and Cardiovascular Surgery c Volume 138, Number 5
Congenital Heart Disease
FIGURE 4. Among the patients who received follow-up catherization after
biventricular repair (n ¼ 17), the right atrial (RA) pressure is negatively
CHD
correlated with the tricuspid valve (TV) size after biventricular repair. The
Z-value was measured by echocardiography when the patient received fol-
low-up catheterization.
the RVP/LVP. This finding was never been well shown in
previous studies, and some texts state that ‘‘there is no evi-
dence that disproportionate enlargement occurs as growth
of the child proceeds, no matter what the surgical proce-
dure.’’17 Even in the recent study by Bryant and associ-
ates,18 RV sinus myectomy was a proposed strategy for
early biventricular repair. Although the RV size increased,
the Z-value of the tricuspid valve did not change apprecia-
bly. However, the Z-value they compared (before and after
myectomy) started at the median age of 1.25 years, not the
neonatal periods, as in our cases.
Because of the rarity of PA–IVS, single institution expe-
rience is generally limited by the small number of cases; the
two largest studies are Congenital Heart Surgeons Society
studies.1,19 However, these studies did not provide data
about late tricuspid valve diameter Z-values. Hanley and co-
workers1 summarized 53 patients from 4 studies.2,3,8,20 The
Z-values decreased without RV outflow tract procedures,
and in patients with RV outflow tract procedures, the overall
Z-value remained unchanged. It is of note that 15% of these
patients experienced a Z-value change ofþ2 and 10% expe-
rienced a change of þ4, but this predictor of tricuspid valve
growth was not known at the time of these studies.
Similarly, we also found that the mean overall Z-value did
not change significantly, but the Z-value change was quite
variable among individual patient. We also found that lower
RVP and smaller initial Z-values were associated with
greater increases in Z-values at their definitive repair.
Z * !6.3
(>60%)
14 RVP and Growth of Tricuspid Valve
We looked for similar findings in other studies. In the
report by Bull and associates21 in 1994, half of the
1163
 Congenital Heart Disease Huang et al

TABLE 3. The changes of Z-value of the tricuspid valve among the

patients with pulmonary atresia–intact ventricular septum by the
right ventricular pressure after decompression procedure
Hypertensive RV Hypotensive RV
Increase in Z-value 6 6
Decrease in Z-value 8 3
No change in Z-value 1 1
Hypertensive RV, Suprasystemic right ventricular pressure; hypotensive RV, right ven-
tricular pressure lower than systemic pressure. (Adapted from Bull et al21 [Figure 5].)

decompressed RVs achieved tricuspid valve growth dispro-

portionate to somatic growth. Reviewing the presented
data,21 there were 15 patients with hypertensive RVs (supra-
systemic RVP) and 10 patients with hypotensive RVs after
RV decompression. The Z-value enlarged in only 40% of
the hypertensive RVs but in 60% of hypotensive RVs.
Thus, hypotensive RVs tended to have better tricuspid valve
growth, although this result did not reach statistical signifi-
CHD

cance (Table 3).

We combined the reports from Patel,3 Shaddy,10 and their
associates, in which the RVP and tricuspid valve diameter
were presented. We included patients with RV–pulmonary
artery continuity and recalculated the Z-values and the
changes in Z-values (n ¼ 26, one outlier was excluded).
We found that the change in Z-value was negatively corre-
lated with postoperative RVP (r ¼!0.552; P ¼ .003) and ini-
tial tricuspid valve Z-value (r ¼!0.579; P ¼ .002) (Figure 5).
These data support our finding that ‘‘disproportionate’’
tricuspid valve growth occurred and that lower RVP and
smaller initial Z-value were predictors for better tricuspid
valve growth.
The mechanisms of RV and tricuspid valve growth are not
clearly understood. Experimental pulmonary stenosis in fe-
tal lambs induced either greatly reduced or markedly in-
creased RV volume. Ventricular enlargement appears to be
associated with a large ventricular volume load resulting
from tricuspid valve regurgitation. A small competent tricus-
pid valve is associated with reduced flow through the ventri-
cle owing to outflow obstruction and a small RV.22 This
experiment suggests that cardiac chamber development is
influenced by blood flow, at least in the prenatal period. In
our study, lower RVP indicated a well-opened outflow tract
and, thus, increased flow through the RV; better tricuspid
valve growth in patients with lower RVP is comparable
with the flow-mediated growth theory. On the other hand,
decreased RVP is beneficial for coronary perfusion to the
RV myocardium, regression of RV myocardial hypertrophy,
and prevention of RV fibrosis. Furthermore, this mechanism
possibly explains the better RV and tricuspid valve growth.
Initial Z-Value Relationship to Tricuspid Valve
Growth
Another interesting finding of our study is that smaller
Z-values were associated with more pronounced Z-value
FIGURE 5. The change in tricuspid valve Z-value in the patients reported
by Shaddy,10 Patel,3 and their associates. A, The higher right ventricular
(RV) pressure after initial decompression procedure is negatively correlated
with tricuspid valve growth. B, The change in Z-value is also negatively cor-
related with the initial tricuspid valve size (Initial Z-value). (Adapted from
Patel et al3 [Tables 1 and 2] and Shaddy et al10 [Table 2].)
increments. This finding is also consistent with other find-
ings in the literature. Percutaneous valvotomy was selec-
tively performed for patients with larger tricuspid valves.
In the reports by both Humpl and associates5 (mean Z-value
¼ !1.33 " 1.58) and Ovaert and colleagues23 (Z-value ¼
!0.8; range: !1.3 to !0.4), the Z-values did not increase
and even decreased after percutaneous pulmonary valvot-
omy. In the surgical series by Shaddy and coauthors,10 the
initial Z-value was !4 " 4, (73% " 21% of the normal
mean), and these authors reported disproportional growth
of the tricuspid valve in the majority of their patients. On
the basis of these findings, we believe that the overall Z-
value would not change if we included all patients, as in
the previous reports,1,14 and disproportionate growth was
possibly more frequently observed in patients with small
initial Z-values.
Z-Value of the Biventricular Circulation
The definite tricuspid valve Z-value criterion for biven-
tricular repair was not well established. Among the 27

1164 The Journal of Thoracic and Cardiovascular Surgery c November 2009

Huang et al
patients who received biventricular repair, 13 (48%) had an
initial Z-value less than!6.3, and the tricuspid valve size in-
creased to more than!6.3 in 7 of them and between!6.3 and
!8.5 in the remaining 4 (Table 2) If we arbitrarily set the
Z-value greater than !6.3 as the criterion for biventricular
repair, there will be 7 patients who became candidates for
biventricular repair despite the initial Z-value being less
than !6.3. We suggest not abandoning biventricular repair
in the neonatal period solely by the echocardiographic Z-
value of the tricuspid valve. In a recent report, Bryant and
coworkers18 from Cleveland suggested a Z-value greater
than!5 as the criterion for biventricular repair. In our series,
inasmuch as we have a tendency to favor biventricular
repair, the Z-value is smaller than in the series by Bryant
and coworkers.18
Follow-up Results
In our study as in other reports,10,15 the majority of pa-
tients underwent biventricular repair when adequate growth
of the tricuspid valve and RV were attained. The follow-up
catheterizations showed that right atrial pressure was within
the normal range, except in 1 patient with pulmonary vein
stenosis, which excluded Fontan circulation. Serial echocar-
diography showed a further increase (mean: 1.14 " 1.09) in
tricuspid valve Z-value after biventricular repair. The find-
ings suggest that the growth potential of the RV and tricus-
pid valve is present in some patients, which makes
a biventricular repair attractive despite the fact that the tri-
cuspid valve is initially too small to support pulmonary cir-
culation in the neonatal period. Although patient survival in
our study was good, on long-term follow-up, patients with
small tricuspid valves might have higher systemic venous
pressures and late atrial arrhythmia complications. The RV
compliance after staged RV decompression was not com-
pletely normal24 and resulted in ‘‘restrictive’’ RV physiol-
ogy.25 Further study is required to further clarify the best
stratification for patients to undergo one, two, or one-and-
a-half ventricle repair.
Study Limitations
This study was retrospective and had a limited number of
cases from a single center. The possibility that the Z-value
change is due to the ‘‘regression to the mean’’ phenomenum
or due to measurement error in a small neonate, which re-
sults in errors of Z-values, could not be completely ex-
cluded. Because the management strategies for PA–IVS
vary from center to center, a multicenter study is required
to determine whether the findings stand in patients who
are treated with other surgical strategies.
CONCLUSIONS
We showed that disproportional growth of the tricuspid
valve occurred, especially in patients with initially small
tricuspid valves and lower RVP after decompression. The
The Journal of Thoracic and Cardiovascular Surgery c Volume 138, Number 5
Congenital Heart Disease
findings support the possibility of a neonate with a small tri-
cuspid valve undergoing biventricular repair after RV de-
compression surgery.
We thank Dr. Fu-Chang Hu, National Center of Excellence for
General Clinical Trial and Research, National Taiwan University
Hospital and College of Public Health, National Taiwan Univer-
sity, for his help in the statistical analysis.
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